The nevoid basal cell carcinoma (Gorlin) syndrome (NBCCS) is an autosomal dominant disorder characterized by multiple developmental defects and cancer susceptibility, in particular to basal cell carcinoma. The human homologue of Drosophila patched (PTCH) was recently identified, mapped to the NBCCS locus on chromosome 9q22.3, and found mutated in patients with NBCCS and also in sporadic basal c...

The lesion traditionally known as odontogenic keratocyst has been renamed by WHO in 2005, as “keratocystic odontogenic tumor” as it is more appropriate and reflects its potential for local, destructive behavior. It is a benign intraosseous neoplasm of jaw, which is unusual due to its characteristic histopathological and clinical features, including potentially aggressive behavior, high recurren...

During a routine faculty prosection of the head and neck region of an adult female cadaver, a partial ossification of the falx cerebri was found. Ossification of other dural areas or regions of the body were not found. In addition, the brain and remaining organs appeared to be grossly normal. Reports of partial ossification of the falx cerebri are still rare and while certain pathologies such a...

エナメル上皮腫は強い局所浸潤性を有し，時には再発や骨破壊も伴う傾向のある歯原性上皮由来の比較的稀な歯原性腫瘍である。われわれは母斑基底細胞癌症候群（nevoid basal cell carcinoma syndrome；NBCCS）の初発症状がエナメル上皮腫によるものであったと考えられる1例を経験したので報告する。患者は特に既往歴と家族歴の無い15歳男性。下顎のエナメル上皮腫を摘出，術後6年後に左側上顎に含歯性囊胞，術後9年後に右側上顎に歯原性角化囊胞を発症した。その後の全身検索にてNBCCSと診断した。術後12 年経過した現在，再発は認めず経過観察をしている。 多様な症候を呈するNBCCSでは顎顔面領域においてもその病態は歯原性角化囊胞などの主要症候だけではない。NBCCSについての疾患理解を深め，早期診断に努めることが重要であると考えられた。

Gorlin's or nevoid basal cell carcinoma syndrome (NBCCS) causes predisposition to basal cell carcinoma (BCC), the commonest cancer in adult human. Mutations in the tumor suppressor gene PTCH1 are responsible for this autosomal dominant syndrome. In NBCCS patients, as in the general population, ultraviolet exposure is a major risk factor for BCC development. However these patients also develop B...

NHNA: nevoid hyperkeratosis of the nipple and/or areola INTRODUCTION Nevoid hyperkeratosis of the nipple and/or areola (NHNA) is an uncommon skin disease without welldefined etiology, reported for the first time in 1923. The disease is characterized by slowly growing verrucous thickening and brown pigmentation of the areola or nipple. Here we report a lesion on the nipple clinically and dermosc...